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Revista Mexicana de Oftalmología

Anales de la Sociedad Mexicana de Oftalmología y Archivos de la Asociación Para Evitar la Ceguera en México
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2008, Number 3

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Rev Mex Oftalmol 2008; 82 (3)

Enfermedad de Stargardt con fondo flavimaculatus y degeneración de la capa de fibras nerviosas de la retina semejando glaucoma. Presentación de un caso y su progresión en diez años

Cubas-Lorenzo V, Gutiérrez JC, López-Ramos L
Full text How to cite this article

Language: Spanish
References: 7
Page: 179-183
PDF size: 266.97 Kb.


Key words:

Stargardt’s disease, fundus flavimaculatus, macular dystrophy, glaucoma.

ABSTRACT

Introduction: Stargardt’s disease and fundus flavimaculatus are variants of the same nosological entity; they are the most common juvenile macular dystrophy and a common cause of central vision loss in adults under the age of 50. It is inherited as an autosomal recessive trait, the age of onset is during the first or second decade of life, progression is rapid and visual prognosis is poor.
Case report: A twenty three year old male diagnosed with Stargardt’s disease and fundus flavimaculatus at age thirteen presents with very low visual capacity and both optic nerves with characteristics of glaucomatous damage. We present the clinical findings and damage progression in posterior pole and fluorescein angiography at the time of diagnosis and ten years later.
Discussion: Nerve fiber layer loss has been observed within multiple pathologies that cause damage to the outer retinal layers, this process can generate changes in the optic nerve appearance which may be confused with glaucoma.
Conclusion: Most macular dystrophies share the same clinical findings, however, their course and prognosis can be quite different.


REFERENCES

  1. Dresner K. Retina and Vitreous. Macular Distrophies. En: Yanoff M, Duker JS (ed). Ophthalmology. 2a. ed. Mosby, España, 2004.

  2. American Academy Of Ophthalmology. Hereditary Retinal and Choroidal Dystrophies. En: American Academy Of Ophthalmology (ed). Basic and Clinical Sciense Course 2006-2007. Section 12: Retina and Vitreous. Singapore, 2006.

  3. Kanski J. Oftalmología Clínica. 5a. ed. Elsevier, España, 2004.

  4. Jiménez-Sierra JM, Ogden T, Van Boemel G. Inherited Retinal Diseases: A diagnostic Guide. St. Louis, Missouri: Mosby, 1989.

  5. Newman N, Stevens R, Heckenlively J. Nerve fiber layer loss in diseases of the outer retinal layer. Brit J Ophthalmol 1987; 71:21-26.

  6. Wirtschafter J. Optic Nerve Axons and Acquired Alterations in the Appearance of the Optic Disc. Tr Am Ophth Soc; LXXXI, 1983.

  7. James M. Optic nerve head structure in glaucoma: astrocytes as mediators of axonal damage. Eye 2000; 14(Pt 3B):437-44.




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Rev Mex Oftalmol. 2008;82